Diagnosis And Treatment In Rheumatology

Scleroderma

Author(s): Małgorzata Wisłowska

Pp: 123-142 (20)

DOI: 10.2174/9781681086552118010010

* (Excluding Mailing and Handling)

Abstract

Systemic sclerosis (SSc) is a connective tissue disease characterized by fibrosis of the skin and internal organs, changes in the microvasculature and in cellular and humoral immunity. The types of disorders include: localized scleroderma (morphea, linear scleroderma, en coup de sabre) and systemic sclerosis (diffuse cutaneous systemic sclerosis [dsSSc], limited cutaneous systemic sclerosis [lcSSc] and systemic sclerosis sine scleroderma). LcSSc is a milder form with slow progression of skin involvement and visceral complications after 10-15 years, but with serious pulmonary arterial hypertension. DsSSc has fast progression of skin sclerosis, from weeks to months, and includes injuries to organs such as the lung, heart and kidney, which may lead to organ failure. The presence of ANA was found in more than 90% of patients with SSc, anti-Scl-70 antibodies in patients with dcSSc in 30-40%, anticentromere antibodies (ACA) in patients with lcSSc in 80-90%. Fibrosis is the end-stage representation of SSc pathogenesis. Severe forms of the disease and rapidly progressive diffuse SSc with pulmonary, cardiac, and renal involvement, are associated with very high mortality rates, estimated at 40-50% in 5 years. No treatment effective therapy exists to stop fibrosis and disease progression beside autologous haemopoietic stem cell transplantation (AHSCT). Cyclophospamide has moderate efficacy in patients with ILD. Methotrexate may be used for the treatment of arthritis. Oesophageal dysmotility and reflux are treated with promotility agents, and proton pump inhibitors. In PAH endothelin receptor antagonists, with PDE5 inhibitor and prostaglandins are used. The most important manifestation is scleroderma renal crisis and is now treated using ACE inhibitors.


Keywords: ACE inhibitors, AHSCT, ANA, Anticentromere antibodies (ACA), Anti-RNA polymerases II and III antibodies, Anti-Scl-70 antibodies, Calcinosis, CREST syndrome, Cyclophospamide, Diffuse cutaneous systemic sclerosis (dsSSc), Endothelin receptor antagonists, Fibrosis, Limited cutaneous systemic sclerosis (lcSSc), Localized scleroderma, Oesophageal dysmotility, PAH, PDE5 inhibitor, Prostaglandins, Proton pump inhibitors, Raynaud’s phenomenon, Sclerodactyly, Systemic sclerosis (SSc), Teleangiectasis, TGFβ.

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      • 12. app/Http/Controllers/ChapterController.php:1465
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      • 11. app/Models/ContentAccess.php:647
      • 12. app/Http/Controllers/ChapterController.php:1478
      • 13. vendor/laravel/framework/src/Illuminate/Routing/Controller.php:54
      • 14. vendor/laravel/framework/src/Illuminate/Routing/ControllerDispatcher.php:43
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      • 13. app/Models/UserAccess/UserAccess.php:1266
      • 14. app/Http/Controllers/ChapterController.php:1518
      • 15. vendor/laravel/framework/src/Illuminate/Routing/Controller.php:54
      • 16. vendor/laravel/framework/src/Illuminate/Routing/ControllerDispatcher.php:43
      • 17. vendor/laravel/framework/src/Illuminate/Routing/Route.php:260
    • (select j.user_access_id,j.volume_id,j.user_id,j.from_ip,j.to_ip,j.type,j.track,"Trial Access" as access_base_text, CASE j.type WHEN 111 THEN true ELSE false END as terms_popup, "S" as content_download_type, null as restricted_user_access_key, null as restricted_user_access_type, CASE j.track WHEN 0 THEN false ELSE true END as tracksAccessByVolume,"chapter" as access_level, j.chapter_id from `user_access_chapter_trail` as `j` inner join `user_access_info` as `info` on `j`.`user_access_id` = `info`.`user_access_id` where ((j.from_ip <= 51739267 and j.to_ip >=51739267 )) and ((j.from_date <= 1740496199 and j.to_date >= 1740496199 and j.perpetual= 0) or (j.from_date is null and j.to_date is null and j.perpetual= 1)) and j.chapter_id = 12264) union (select j.user_access_id,j.volume_id,j.user_id,j.from_ip,j.to_ip,j.type,j.track,"Subscribed" as access_base_text, false as terms_popup, "S" as content_download_type, null as restricted_user_access_key, null as restricted_user_access_type, CASE j.track WHEN 0 THEN false ELSE true END as tracksAccessByVolume,"chapter" as access_level, j.chapter_id from `user_access_chapter_corporate` as `j` inner join `user_access_info` as `info` on `j`.`user_access_id` = `info`.`user_access_id` where ((j.from_ip <= 51739267 and j.to_ip >=51739267 )) and ((j.from_date <= 1740496199 and j.to_date >= 1740496199 and j.perpetual= 0) or (j.from_date is null and j.to_date is null and j.perpetual= 1)) and j.chapter_id = 12264) union (select j.user_access_id,j.volume_id,j.user_id,j.from_ip,j.to_ip,j.type,j.track,"Token Based Access" as access_base_text, false as terms_popup, "R" as content_download_type, j.uc_id as restricted_user_access_key, "v" as restricted_user_access_type, CASE j.track WHEN 0 THEN false ELSE true END as tracksAccessByVolume,"chapter" as access_level, j.chapter_id from `user_access_chapter_token` as `j` inner join `user_access_info` as `info` on `j`.`user_access_id` = `info`.`user_access_id` where ((j.from_ip <= 51739267 and j.to_ip >=51739267 )) and ((j.from_date <= 1740496199 and j.to_date >= 1740496199 and j.perpetual= 0) or (j.from_date is null and j.to_date is null and j.perpetual= 1)) and j.chapter_id = 12264)
      820μsalphaeurekaselec_live_10_06_2022UserAccess.php#1266
      Backtrace
      • 13. app/Models/UserAccess/UserAccess.php:1266
      • 14. app/Http/Controllers/ChapterController.php:1549
      • 15. vendor/laravel/framework/src/Illuminate/Routing/Controller.php:54
      • 16. vendor/laravel/framework/src/Illuminate/Routing/ControllerDispatcher.php:43
      • 17. vendor/laravel/framework/src/Illuminate/Routing/Route.php:260
    • select b.banner_link, b.banner_name, b.banner_script, b.banner_remarks, b.banner_file_name, b.banner_placement, bd.content_type, bd.content_id from `tbl_banner` as `b` inner join `tbl_banner_detail` as `bd` on `bd`.`banner_id` = `b`.`banner_id` inner join `users` as `u` on `u`.`id` = `b`.`updated_by` where `b`.`banner_ebook` = 1 and `bd`.`content_type` = 'B' and `bd`.`content_id` = 2460 and `b`.`banner_from_date` <= 1740496199 and `b`.`banner_to_date` >= 1740496199 and `b`.`banner_status` = 'A' order by `b`.`banner_id` desc
      1.09msalphaeurekaselec_live_10_06_2022Banner.php#95
      Bindings
      • 0: 1
      • 1: B
      • 2: 2460
      • 3: 1740496199
      • 4: 1740496199
      • 5: A
      Backtrace
      • 13. app/Models/Banner.php:95
      • 14. app/Http/helpers.php:404
      • 17. vendor/laravel/framework/src/Illuminate/Filesystem/Filesystem.php:124
      • 18. vendor/laravel/framework/src/Illuminate/View/Engines/PhpEngine.php:58
      • 19. vendor/laravel/framework/src/Illuminate/View/Engines/CompilerEngine.php:73
    • select count(*) as aggregate from `uc_cart_products` where cart_id='7d99794d4b022d025619b55524203abc'
      230μsalphaeurekaselec_live_10_06_2022Cart.php#271
      Backtrace
      • 15. app/Models/Cart.php:271
      • 16. view::layouts._header:260
      • 18. vendor/laravel/framework/src/Illuminate/Filesystem/Filesystem.php:124
      • 19. vendor/laravel/framework/src/Illuminate/View/Engines/PhpEngine.php:58
      • 20. vendor/laravel/framework/src/Illuminate/View/Engines/CompilerEngine.php:73
    App\Models\Keywords
    24Keywords.php#?
    App\Models\Chapter
    1Chapter.php#?
    App\Models\Ebook
    1Ebook.php#?
        _token
        w6HLZjxKedyubhtc9fXsoKqOAiyrAS4P3LuJpNsP
        uc_cart_id
        7d99794d4b022d025619b55524203abc
        _previous
        array:1 [ "url" => "http://alpha.eurekaselect.com/chapter/12264" ]
        _flash
        array:2 [ "old" => [] "new" => [] ]
        PHPDEBUGBAR_STACK_DATA
        []
        path_info
        /chapter/12264
        status_code
        200
        
        status_text
        OK
        format
        html
        content_type
        text/html; charset=UTF-8
        request_query
        []
        
        request_request
        []
        
        request_headers
        0 of 0
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        request_cookies
        0 of 0
        array:1 [ "wayfindertarget" => null ]
        response_headers
        0 of 0
        array:5 [ "content-type" => array:1 [ 0 => "text/html; charset=UTF-8" ] "cache-control" => array:1 [ 0 => "no-cache, private" ] "date" => array:1 [ 0 => "Tue, 25 Feb 2025 15:09:59 GMT" ] "set-cookie" => array:2 [ 0 => "XSRF-TOKEN=eyJpdiI6ImpWR2hGMjN5Sk5pUDhhTVJ1ZStEbWc9PSIsInZhbHVlIjoiQVNTY0lZZzBlbmJ1c3BXVEFEbGNRWU45NThhUmsyZmFIMEtKbTRQa3U1dkg5QXRpK01JczY1OXFNeGc0V2JQcjlwT2pLYlhhbEpaRUk3RDk1Z1dBRkwwblY5YUU3dzlxRmMvVXIycDY1NTlUNnhoaEFvOFVEUGRwZi9VaTdDYmoiLCJtYWMiOiI1N2RmYTAyOTRjOGM2ZmEzOGU5YmNkMTlkMzYwMGUyMmY3NWYxMjBjMzhmNjdhMjQ4ZjRmMjIwYzc0ZmU1ZDMwIiwidGFnIjoiIn0%3D; expires=Fri, 28 Feb 2025 03:09:59 GMT; Max-Age=216000; path=/; secure; samesite=laxXSRF-TOKEN=eyJpdiI6ImpWR2hGMjN5Sk5pUDhhTVJ1ZStEbWc9PSIsInZhbHVlIjoiQVNTY0lZZzBlbmJ1c3BXVEFEbGNRWU45NThhUmsyZmFIMEtKbTRQa3U1dkg5QXRpK01JczY1OXFNeGc0V2JQcjlwT2pLY" 1 => "alphaeurekaselectnet_session=eyJpdiI6IlNKMm9LZzFCaXlOaVFCOWh1eHdkRFE9PSIsInZhbHVlIjoid0hTNVFweG1CQ0xzV1lQSGtzMVNVZWphRk9qV2Q1RWZkZjVFUkkyWXNjem9pbXBVUzhCVnp1czJYaWRkVmxRT2dYY2lkUDNQVUJ3eXJOSWJhVjZpSCtldW91SXNRWS9OSHFnK0Z5VWNiSWliK2FHUFNLYUdCcnRuVTFFZWdlYmYiLCJtYWMiOiJlOTRkNjQ5NmQ0MGFkODc2ZmM5NjVmYTFmZmFhMmMwN2VhNDhkMGNhMjNhMDRiNGIxODUxYTk2NTIwMjIxNTVlIiwidGFnIjoiIn0%3D; path=/; secure; httponly; samesite=laxalphaeurekaselectnet_session=eyJpdiI6IlNKMm9LZzFCaXlOaVFCOWh1eHdkRFE9PSIsInZhbHVlIjoid0hTNVFweG1CQ0xzV1lQSGtzMVNVZWphRk9qV2Q1RWZkZjVFUkkyWXNjem9pbXBVUzhCVnp1czJ" ] "Set-Cookie" => array:2 [ 0 => "XSRF-TOKEN=eyJpdiI6ImpWR2hGMjN5Sk5pUDhhTVJ1ZStEbWc9PSIsInZhbHVlIjoiQVNTY0lZZzBlbmJ1c3BXVEFEbGNRWU45NThhUmsyZmFIMEtKbTRQa3U1dkg5QXRpK01JczY1OXFNeGc0V2JQcjlwT2pLYlhhbEpaRUk3RDk1Z1dBRkwwblY5YUU3dzlxRmMvVXIycDY1NTlUNnhoaEFvOFVEUGRwZi9VaTdDYmoiLCJtYWMiOiI1N2RmYTAyOTRjOGM2ZmEzOGU5YmNkMTlkMzYwMGUyMmY3NWYxMjBjMzhmNjdhMjQ4ZjRmMjIwYzc0ZmU1ZDMwIiwidGFnIjoiIn0%3D; expires=Fri, 28-Feb-2025 03:09:59 GMT; path=/; secureXSRF-TOKEN=eyJpdiI6ImpWR2hGMjN5Sk5pUDhhTVJ1ZStEbWc9PSIsInZhbHVlIjoiQVNTY0lZZzBlbmJ1c3BXVEFEbGNRWU45NThhUmsyZmFIMEtKbTRQa3U1dkg5QXRpK01JczY1OXFNeGc0V2JQcjlwT2pLY" 1 => "alphaeurekaselectnet_session=eyJpdiI6IlNKMm9LZzFCaXlOaVFCOWh1eHdkRFE9PSIsInZhbHVlIjoid0hTNVFweG1CQ0xzV1lQSGtzMVNVZWphRk9qV2Q1RWZkZjVFUkkyWXNjem9pbXBVUzhCVnp1czJYaWRkVmxRT2dYY2lkUDNQVUJ3eXJOSWJhVjZpSCtldW91SXNRWS9OSHFnK0Z5VWNiSWliK2FHUFNLYUdCcnRuVTFFZWdlYmYiLCJtYWMiOiJlOTRkNjQ5NmQ0MGFkODc2ZmM5NjVmYTFmZmFhMmMwN2VhNDhkMGNhMjNhMDRiNGIxODUxYTk2NTIwMjIxNTVlIiwidGFnIjoiIn0%3D; path=/; secure; httponlyalphaeurekaselectnet_session=eyJpdiI6IlNKMm9LZzFCaXlOaVFCOWh1eHdkRFE9PSIsInZhbHVlIjoid0hTNVFweG1CQ0xzV1lQSGtzMVNVZWphRk9qV2Q1RWZkZjVFUkkyWXNjem9pbXBVUzhCVnp1czJ" ] ]
        session_attributes
        0 of 0
        array:5 [ "_token" => "w6HLZjxKedyubhtc9fXsoKqOAiyrAS4P3LuJpNsP" "uc_cart_id" => "7d99794d4b022d025619b55524203abc" "_previous" => array:1 [ "url" => "http://alpha.eurekaselect.com/chapter/12264" ] "_flash" => array:2 [ "old" => [] "new" => [] ] "PHPDEBUGBAR_STACK_DATA" => [] ]
        ClearShow all
        Date ↕MethodURLData
        #12025-02-25 15:09:59GET/chapter/122641121926